Coexistence of congenital superior rectus muscle fibrosis and Marcus Gunn jaw-winking phenomenon

Abstract

A 6-year-old male involving a coexistence of congenital fibrosis of the superior rectus muscle (SR) with Marcus Gunn Jaw-Winking phenomenon presented with an elevation of the upper eyelid along with a chronically open mouth that had been present since early infancy. There was extropia and hypertropia, a limitation of elevation and depression in the right eye, and an abnormal head posture (AHP) with a left head tilt, right face turn, and a chin-up head position. He also displayed an upper eyelid retraction and a 2-mm lagophthalos in the right eye. Results of the forced duction test indicated a constricted SR of the right eye. A dissection of the SR from the levator palpebrae superioris and a 5-mm SR recession was performed within the right eye. At 2 months post surgery, there was a 3 prism diopters (PD) hypertropia in the right eye when the left eye was fixated on the target and a 10 PD hypotropia in the left eye when the right eye was fixated on the target, along with an improvement in his AHP. In this rare case with a coexistence of congenital fibrosis of the SR and Marcus Gunn jaw-winking phenomenon, we found that the performance of an individualized surgical protocol proved effective in reducing the vertical deviation and improving AHP.